Related Experiment Videos
Multiple cystic encephalomalacia of infancy: computed tomographic findings in two cases with associated intracerebral
Insights
Two infants developed severe encephalopathy with seizures and decerebration. Early computed tomography (CT) scans showed unique abnormalities, aiding diagnosis of this rare condition.
Area of Science:
- Pediatric Neurology
- Neuroradiology
- Infectious Diseases
Background:
- Acute encephalopathic illnesses in infants can present with severe neurological deficits.
- Differentiating various causes of infant encephalopathy is crucial for management.
- Herpes simplex virus (HSV) is a known cause of neonatal encephalitis.
Observation:
- Two infants presented with acute encephalopathy, seizures, and cerebrospinal fluid (CSF) abnormalities.
- One infant had HSV infection; both showed distinct computed tomography (CT) findings.
- CT scans revealed progressive cortical calcifications and white matter changes, unlike typical meningitis/encephalitis.
Findings:
- The clinical and imaging findings were consistent with multiple cystic encephalomalacia.
- This rare condition led to chronic decerebration in both patients.
- Autopsy confirmed multiple cystic encephalomalacia in one infant.
Implications:
- Multiple cystic encephalomalacia of infancy is a rare condition with a poor prognosis.
- Early diagnosis via CT imaging is vital for clinical counseling and patient management.
- Distinct CT findings can help differentiate this entity from other infant encephalopathies.
Abstract:
Two initially healthy infants developed acute encephalopathic illnesses characterized by stupor, seizures, cerebrospinal fluid (CSF) erythrocytic and monocytic pleocytosis, increased CSF protein, and decreased CSF glucose and progression to chronic decerebration. In one case, herpes simplex virus was recovered from cutaneous lesions. The initial computed tomography (CT) scan revealed widespread subcortical increased attenuation with further increase after contrast medium injection and patchy areas of decreased attenuation in the deep cerebral white matter. Subsequent CT scans demonstrated progressive cortical calcifications and persistence of low attenuation areas. Autopsy revealed multiple cystic encephalomalacia. The second infant had similar clinical, CSF, and CT findings but remains in a chronic decerebrate state at 14 months of age. The CT abnormalities seen in these patients have not been encountered in any of 13 other infants with the clinical diagnosis of meningitis or encephalitis. Multiple cystic encephalomalacia of infancy is a rare condition with a uniformly bleak prognosis. Computed tomography may prove useful in the early diagnosis, thereby aiding clinicians in counseling and in the acute and long-term management of patients with this lesion.